Search Results - "BRAULKE, T"

Lysosomal dysfunction causes neurodegeneration in mucolipidosis II 'knock-in' mice by KOLLMANN, K, DAMME, M, KÄKELÄ, R, WALKLEY, S. U, BRAULKE, T, MARKMANN, S, MORELLE, W, SCHWEIZER, M, HERMANS-BORGMEYER, I, RÖCHERT, A. K, POHL, S, LÜBKE, T, MICHALSKI, J.-C

“…Mucolipidosis II is a neurometabolic lysosomal trafficking disorder of infancy caused by loss of mannose 6-phosphate targeting signals on lysosomal proteins,…”
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The role of the IGF axis in hepatocarcinogenesis by Scharf, J-G, Braulke, T

“…Primary hepatocellular carcinoma (HCC) is one of the most common forms of malignant cancer with the fourth highest mortality rate worldwide. Major risk factors…”
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Molecular analysis of the GlcNac-1-phosphotransferase by Braulke, T., Pohl, S., Storch, S.

“…Summary Modification of the carbohydrate chains of soluble lysosomal enzymes with mannose 6-phosphate residues is a prerequisite for their mannose 6-phosphate…”
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Membrane translocation of glutaric acid and its derivatives by Mühlhausen, C., Burckhardt, B. C., Hagos, Y., Burckhardt, G., Keyser, B., Lukacs, Z., Ullrich, K., Braulke, T.

“…Summary The neurodegenerative disorder glutaric aciduria type I (GA I) is characterized by increased levels of cytotoxic metabolites such as glutaric acid (GA)…”
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Multiple post-translational modifications of mouse insulin-like growth factor binding protein-6 expressed in epithelial Madin–Darby canine kidney cells by Shalamanova, L., Kübler, B., Storch, S., Scharf, J.-G., Braulke, T.

“…Insulin-like growth factors (IGFs), IGF receptors and IGF binding proteins (IGFBPs) participate in the regulation of proliferation and differentiation of…”
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P44.13: Prenatal diagnosis of mucolipidosis II using genetic testing by Hartung, J., Pötschick, K., Kunz, J., Kalache, K., Braulke, T.

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Synthesis of insulinlike growth factor binding proteins and of the acid-labile subunit in primary cultures of rat hepatocytes, of Kupffer cells, and in cocultures: Regulation by insulin, insulinlike growth factor, and growth hormone by Scharf, J, Ramadori, G, Braulke, T, Hartmann, H

“…The adult liver is the main source of circulating insulinlike growth factors (IGFs) and their serum binding proteins (IGFBPs) including the acid-labile subunit…”
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Synthesis of insulinlike growth factor binding proteins and of the acid‐labile subunit in primary cultures of rat hepatocytes, of Kupffer cells, and in cocultures: Regulation by insulin, insulinlike growth factor, and growth hormone by Scharf, J, Ramadori, G, Braulke, T, Hartmann, H

“…The adult liver is the main source of circulating insulinlike growth factors (IGFs) and their serum binding proteins (IGFBPs) including the acid‐labile subunit…”
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Vascular dysfunction as an additional pathomechanism in glutaric aciduria type I by Mühlhausen, C., Ergün, S., Strauss, K. A., Koeller, D. M., Crnic, L., Woontner, M., Goodman, S. I., Ullrich, K., Braulke, T.

“…The metabolic hallmark of glutaric aciduria type I (GA I) isthe deficiency of glutaryl‐CoA dehydrogenase (GCDH) with subsequent accumulation of glutaric acid,…”
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Type-2 IGF receptor: a multi-ligand binding protein by Braulke, T

“…The Type-2 insulin-like growth factor receptor (IGF2R) is a ubiquitously expressed integral glycoprotein with a molecular mass of 300 kDa. Four different…”
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Origin of lysosomal proteins by Braulke, T

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Effects of brefeldin A on the endocytic route. Redistribution of mannose 6-phosphate/insulin-like growth factor II receptors to the cell surface by DAMKE, H, KLUMPERMAN, J, VON FIGURA, K, BRAUKLE, T

“…The effect of brefeldin A (BFA) on the trafficking of the mannose 6-phosphate/insulin-like growth factor II receptor within the endocytic route was analyzed…”
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Alteration in pancreatic immunoreactivity of insulin-like growth factor (IGF)-binding protein (IGFBP)-6 and in intracellular degradation of IGFBP-3 in fibroblasts of IGF-II receptor/IGF-II-deficient mice by Braulke, T, Dittmer, F, Götz, W, von Figura, K

“…The Type-2 insulin-like growth factor receptor (IGF2R) mediates the transport of lysosomal hydrolases to lysosomes and the clearance of insulin-like growth…”
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In vitro phosphorylation of the 46-kDa mannose 6-phosphate receptor by casein kinase II. Structural requirements for efficient phosphorylation by KÖRNER, C, HERZOG, A, WEBER, B, ROSORIUS, O, HEMER, F, SCHMIDT, B, BRAULKE, T

“…Some steps in the receptor-mediated transport of newly synthesized mannose 6-phosphate-containing lysosomal enzymes are assumed to be accompanied by changes in…”
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Role of protein phosphatases in insulin-like growth factor II (IGF II)-stimulated mannose 6-phosphate/IGF II receptor redistribution by BRAULKE, T, MIESKES, G

“…The regulated expression of mannose 6-phosphate/insulin-like growth factor II (M6P/IGF II) receptors in plasma membranes has previously been shown to be…”
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Phosphorylation of the human 46-kDa mannose 6-phosphate receptor in the cytoplasmic domain at serine 56 by Hemer, F, Körner, C, Braulke, T

“…The human 46-kDa mannose 6-phosphate receptor (MPR46) is phosphorylated in its cytoplasmic domain at serine residues. Substitution of cytoplasmic serines (at…”
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The Lysosomal Protein Arylsulfatase B Is a Key Enzyme Involved in Skeletal Turnover by Pohl, Sandra, Angermann, Alexandra, Jeschke, Anke, Hendrickx, Gretl, Yorgan, Timur A, Makrypidi‐Fraune, Georgia, Steigert, Anita, Kuehn, Sonja C, Rolvien, Tim, Schweizer, Michaela, Koehne, Till, Neven, Mona, Winter, Olga, Velho, Renata Voltolini, Albers, Joachim, Streichert, Thomas, Pestka, Jan M, Baldauf, Christina, Breyer, Sandra, Stuecker, Ralf, Muschol, Nicole, Cox, Timothy M, Saftig, Paul, Paganini, Chiara, Rossi, Antonio, Amling, Michael, Braulke, Thomas, Schinke, Thorsten

“…ABSTRACT Skeletal pathologies are frequently observed in lysosomal storage disorders, yet the relevance of specific lysosomal enzymes in bone remodeling cell…”
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Human lysosomal acid phosphatase is transported as a transmembrane protein to lysosomes in transfected baby hamster kidney cells by Waheed, A., Gottschalk, S., Hille, A., Krentler, C., Pohlmann, R., Braulke, T., Hauser, H., Geuze, H., Figura, K.

“…BHK cells transfected with human lysosomal acid phosphatase (LAP) cDNA (CT29) expressed 70‐fold higher enzyme activities of acid phosphatase than…”
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Mucolipidosis II is caused by mutations in GNPTA encoding the α/β GlcNAc-1-phosphotransferase by Braulke, Thomas, Tiede, Stephan, Storch, Stephan, Lübke, Torben, Henrissat, Bernard, Bargal, Ruth, Raas-Rothschild, Annick

“…Mucolipidosis II (ML II) is a fatal lysosomal storage disorder resulting from defects in the multimeric GlcNAc-1-phosphotransferase responsible for the initial…”
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Metabolic actions of insulin-like growth factor II in cultured adult rat hepatocytes are not mediated through the insulin-like growth factor II receptor by HARTMANN, H, MEYER-ALBER, A, BRAULKE, T

“…Short- and long-term regulation of hepatic carbohydrate metabolism by insulin-like growth factor II was studied in primary cultures of adult rat hepatocytes…”
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